Franco E L, de Camargo B, Saba L, Marques L A
Institut Armand-Frappier, Université du Québec, Montreal, Canada.
Int J Cancer. 1991 Jul 9;48(5):641-6. doi: 10.1002/ijc.2910480502.
The epidemiological and clinical features of Wilms' tumor (WT) were analyzed in 176 patients admitted to the Brazilian WT Study Group. The occurrence of congenital anomalies (9.1%) and the M:F ratio (0.83) were comparable to those observed by the US National WT Study and the International Society of Pediatric Oncology trials. Bilateral cases were younger on average than unilateral cases (37.2 vs. 45.0 months). Ethnic group and gender were also associated with age, with non-white children being generally older (46.1 months) than whites (39.5 months), and boys being younger than girls (37.0 vs. 46.1 months). However, the most important factor in association with age at diagnosis was disease stage. No early disease patients were diagnosed after 8 years of age (mean: 37.5 months), whereas 10% of those with advanced disease were diagnosed between the ages of 8 and 10 years (mean: 56.3 months). There were no clear distinctions in age distributions on the basis of the presence of tumor multicentricity, intra- or perilobar nephroblastomatosis, and of a combination of putative genetic determinants. The mean age at diagnosis for cases with congenital defects was higher than that for the remaining patients. Some of the study results are in support of the recessive oncogene model for WT. However, the possible confounding effect of disease stage and the strong interdependence of the remaining factors may be masking important relations in regard to mutational events occurring during embryogenesis.
对巴西威尔姆斯瘤（WT）研究组收治的176例患者的WT流行病学和临床特征进行了分析。先天性异常的发生率（9.1%）和男女比例（0.83）与美国国家WT研究及国际儿科肿瘤学会试验观察到的情况相当。双侧病例的平均年龄比单侧病例小（37.2个月对45.0个月）。种族和性别也与年龄相关，非白人儿童通常比白人儿童年龄大（46.1个月对39.5个月），男孩比女孩年龄小（37.0个月对46.1个月）。然而，与诊断时年龄相关的最重要因素是疾病分期。8岁后未诊断出早期疾病患者（平均：37.5个月），而10%的晚期疾病患者在8至10岁之间被诊断出（平均：56.3个月）。基于肿瘤多中心性、叶内或叶旁肾母细胞瘤病以及假定遗传决定因素的组合，年龄分布没有明显差异。先天性缺陷病例的诊断平均年龄高于其余患者。一些研究结果支持WT的隐性癌基因模型。然而，疾病分期可能的混杂效应以及其余因素的强烈相互依赖性可能掩盖了胚胎发生过程中发生的突变事件的重要关系。
